Wednesday, April 25, 2007

I thought I'd start describing Cicily's health gear with her g-tube. Because Cicily has a very wide cleft palate and an underdeveloped lower jaw, she can't eat well by mouth. In addition, as you can see by the picture of me before she was born, she hasn't had a history of swallowing well (thus the 3 gallons of amniotic fluid she swam in). So when she was 2 weeks old she had a surgery done to place a g-tube so she could eat via a feeding tube into her stomach. She actually used to eat @10mLs of milk/day by mouth, but since she's had pneumonia twice and been sick on and off all winter we don't offer her any more food by mouth so she doesn't have a chance to aspirate it into her lungs. Instead we've been giving her an empty bottle so she associates having something in her mouth with her tummy getting full. She actually loves her bottle- it's the only thing she likes to have in her mouth and she'll hold it and shove it in! Hopefully in the next couple months we can work with her on starting solid food by mouth and we'll see how well she can swallow.

The Button:

The part on the left side of the pic is the "balloon" which is deflated to begin with, then inserted into the stoma (hole) in her stomach and filled with 5 mLs of water to hold it in place. The part on the left side is the "button" which always sits on her stomach and opens up for us to connect the "mickey adapter" when she needs to eat.

How the button looks on her tummy- I always describe it as the blow up thing on a beach ball :)

Button with mickey connected - eating in progress! One convenient thing is Cis can eat while she's sleeping.

Kangaroo pump- breast milk goes from the bag on top through the tube into Cis' tummy

Monday, April 23, 2007

I decided it would be nice to have a little website for Cicily. Since she catches illnesses so easily(via the trach) I think some of her doctors see her more than her extended family and friends do! So I thought this could be a way of letting everyone get to know our wonderful girl and keep up on the fun (and not as fun) things happening in her life.

Hello to everyone! I've been wanting to send out an announcement and some pictures of our new little girl Cicily for a while, but have been a little busy...

Cicily Anne was born on October 3 and was welcomed by Mom and Dad, 6 doctors and 15-20 nurses. The doctors put together a team for a planned c-section in the O.R. because they suspected she had a cleft palate and possibly would have difficulty breathing on her own. Ultrasounds had indicated she had a small lower jaw and wasn't swallowing well as told by the overabundance of amniotic fluid I had (3 gallons according to the doc). She was 2 1/2 weeks early and weighed in at 6 lbs. 12 oz. The doctors intubated her (gave her a breathing tube) right at birth. Then she was whisked off to the NICU. The doctors diagnosed her with Treacher Collins Syndrome. This is a genetic mutation that she inherited from us. Most likely Chris nor I have it, one of our genes just mutated and she happened to score that gene! It causes underdevelopment of the bones in the face and head to varying degrees.

The day after she was born the doctor tried to extubate her(take out her breathing tube), but she couldn't breathe on her own in any position they tried. So a week after her birth she had her first surgery to put in her trach. The E.N.T. (ear, nose and throat dr) said because her chin is small and set back, her tongue sits right on top of her trachea, blocking her airway. So he did a tracheostomy so she could breathe. Her plastic surgeon says at 3-4 years of age they can lengthen her jaw and she should be able to breathe on her own and lose the trach.

Cicily also has a cleft palate (doesn't include a cleft lip). The roof of her mouth is open in the soft tissue area and you can see her nasal bone. This they will repair at 9-12 months of age.With the cleft palate and the trach she has difficulties eating. She actually sucks much better than the doctors thought she would be able to. We give her a bottle with a special nipple for cleft palates about once a day. She can't get nearly enough food that way though because it takes a long time for her to get it out and they're afraid she will aspirate milk into her lungs. Consequently, at 2 weeks of age Cicily had her 2nd surgery for the surgeon to put in a g-tube (feeding tube directly to her stomach).

Another symptom of Treacher Collins Cicily has is small ears and unformed ear canals. They tested Cicily's hearing and she hears 60 decibels (as if you plugged your ears). So she has bone conducting hearing aids that we put on her when she is awake. These activate her existing cochlea in her inner ears through vibration and she can hear.

Now after 3 weeks in the NICU and getting her to be able to breathe, eat and hear, Cicily is home with us! She is doing fabulously and Mom and Dad have almost received RN degrees learning everything they need to to care for her. She is a good sleeper and not a very demanding baby. We just try not to get her angry, because she is a fighter! The doctor that intubated her at birth said she tried to punch him and I was present when 5 nurses had to hold her down after she'd pulled out an I.V. they'd just put in. We figure this will be a good trait as she goes through her other numerous surgeries. Treacher Collins doesn't affect any brain functions, so she should have no problems mentally. From what we understand at this point, all of her surgeries should be done by the time she's 8 years old or so. We just think she'll be a fun, brilliant \ngirl just like her big sister! We're very thankful to all who have said prayers and sent love in her behalf. We've had many little miracles over the last 7 weeks.

Sorry if this is way more info than you cared to read, I just wanted to try and pre-answer any questions. Feel free to ask us if you have any questions or anything, I always like talking about my girl! If there are any family/friends I haven't sent this to that would be interested in Cicily, feel free to send this on.

Addendum to initial email about Cicily's birthI sort of laugh now when I read that email. I'm kinda glad I thought it would all be that simple back then! In reality, it looks like most of her surgeries to get her started will be done by the time she's 6 (cleft palate, jaw distraction, hearing aid implants, ear construction). But many people with TCS have 20-30 surgeries by the time they're 20. So we'll just see how Cicily grows!After seeing Cicily's palate, the plastic surgeon decided she'd probably have to wait until she's more like 1 1/2 to 2 years old before she does the first surgery to repair her cleft. We're also working on getting her jaw distraction done earlier than 3-4 yrs old, more like 2-3. As for her hearing aids, we're also trying to push those to be implanted earlier than usual- like around 1-2 yrs old.Anyway, it seems to be all a matter of just how she grows, Dr.s opinions, insurance companies, and ultimately what her parents think is best.Check back for more updates on surgeries - we'll have plenty of changes I'm sure!!

New to Cicily's blog? Read some FAQs about Cicily:

Cicily at 8 years old

About Cicily

Cicily has degenerative hypomyelination (leukodystrophy) - no white matter coating her brain, which is terminal. For Cicily, this means she has limited motor control and "shakey" movements. It does not affect Cicily's cognitive abilities, as she communicates and learns at a standard level for her age.

Cicily also has Treacher Collins Syndrome. For Cicily this basically means she was born with a cleft palate, small lower jaw, small ears, and no ear canals.

Cis is such a tough girl though, and she's so easy going. She handles all of her difficulties so well. She's certainly up for her challenges! She continues to be a medical mystery and a joyful girl.